Linked Life Data

3745934

Source:http://linkedlifedata.com/resource/pubmed/id/3745934

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1986-10-2
pubmed:abstractText
In order to examine the claim for a numerical and structural abnormality of the hemidesmosomes in junctional epidermolysis bullosa (JEB), a morphometric analysis of unseparated dermal-epidermal junction was undertaken in 11 subjects with JEB. Of these, 5 died in infancy with "lethal" disease, 3 were children still alive at 1-6 years with "indeterminate" disease, and 3 were females aged 20-60 years with variable phenotypic expression of "nonlethal" JEB. All the lethal cases had reduced numbers of hemidesmosomes which were small and lacked normal subbasal dense plates, with the exception of 1 patient whose hemidesmosomes were structurally and numerically normal. The principal hemidesmosome abnormality in the 3 cases with indeterminate JEB was the absence of normal subbasal dense plates. In 2 of the 3 cases of nonlethal JEB, the hemidesmosomes appeared normal, whereas in the third patient they showed a similar abnormality to that present in the majority of the lethal group. These results demonstrate that JEB is an ultrastructurally heterogeneous condition, and suggest that, even though the hemidesmosome abnormalities may be of diagnostic value, they do not correlate sufficiently well with the clinical outcome to be useful as a prognostic indicator.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0022-202X
pubmed:author
pubmed:issnType
Print
pubmed:volume
86
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
51-6
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1986
pubmed:articleTitle
Hemidesmosome heterogeneity in junctional epidermolysis bullosa revealed by morphometric analysis.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't